Archives of disease in childhood
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Rett syndrome is a severe neurodevelopmental disorder of unknown aetiology. A prolonged QT interval has been described previously in patients with Rett syndrome. To investigate QT prolongation and the presence of cardiac tachyarrhythmias in Rett syndrome electrocardiography and 24 hour Holter monitoring were performed prospectively in a cohort of 34 girls with Rett syndrome. ⋯ Compared with a group of healthy controls of a similar age range, the patients with Rett syndrome had significantly longer corrected QT values. Clinical severity was not a predictor for prolonged QT intervals in the Rett syndrome cohort. The prolonged QT syndrome is a serious and potentially lethal cardiac disorder and should be considered in all girls with Rett syndrome.
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To compare the modes of death and factors leading to withdrawal or limitation of life support in a paediatric intensive care unit (PICU) in a developing country. ⋯ Limitation of treatment is the most common mode of death in a developing country's PICU and active withdrawal is still not widely practised. Paediatricians in developing countries are becoming more proactive in managing death and dying but have to consider sociocultural and religious factors when making such decisions.
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To determine prospectively the efficacy of surfactant in acute respiratory distress syndrome. ⋯ Surfactant moderately improves oxygenation in some children with severe acute respiratory distress syndrome secondary to pulmonary or systemic disease.
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Case Reports
Mannitol and frusemide in the treatment of diuretic resistant oedema in nephrotic syndrome.
Three children (two girls aged 7 and 9 years, and one boy aged 4 years) with diuretic resistant oedema in steroid resistant nephrotic syndrome were treated with a combination of intravenous mannitol and frusemide. All three responded with loss of oedema of 10% to 30% of body weight over one week. ⋯ Mannitol-frusemide combination is a safe, inexpensive, and effective treatment for diuretic resistant oedema. Its use in other conditions and in developing countries (where the availability and purity of 20% albumin is limited) needs to be explored.
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Capillary refill time is an important diagnostic adjunct in the acute resuscitation phase of the shocked child. This study assesses its relation to commonly measured haemodynamic parameters in the postresuscitation phase when the child has reached the intensive care unit, and compares this with core-peripheral temperature gap. ⋯ Among ventilated, general intensive care patients, capillary refill time is related weakly to blood lactate and SVI. A normal value for capillary refill time of < or = 2 seconds has little predictive value and might be too conservative for this population; septic shock.